Hematology Reports (Sep 2015)

B-cell-rich T-cell lymphoma associated with Epstein-Barr virus-reactivation and T-cell suppression following antithymocyte globulin therapy in a patient with severe aplastic anemia

  • Nobuyoshi Hanaoka,
  • Shogo Murata,
  • Hiroki Hosoi,
  • Aiko Shimokado,
  • Toshiki Mushino,
  • Kodai Kuriyama,
  • Kazuo Hatanaka,
  • Akinori Nishikawa,
  • Miwa Kurimoto,
  • Takashi Sonoki,
  • Yasuteru Muragaki,
  • Hideki Nakakuma

DOI
https://doi.org/10.4081/hr.2015.5906
Journal volume & issue
Vol. 7, no. 3

Abstract

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B-cell lymphoproliferative disorder (B-LPD) is generally characterized by the proliferation of Epstein-Barr virus (EBV-infected B lymphocytes. We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG) therapy in a patient with aplastic anemia. The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma. This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab.

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